| Literature DB >> 28536823 |
Stefano Gentileschi1, Donato Rigante2, Antonio Vitale1, Jurgen Sota1, Bruno Frediani1, Mauro Galeazzi1, Luca Cantarini3,4.
Abstract
Tumor necrosis factor receptor-associated periodic syndrome (TRAPS), caused by mutations in the TNFRSF1A gene, is the most frequent autosomal dominant autonflammatory disease displaying a relevant risk of reactive AA amyloidosis, if left untreated. Our report deals with one adult with TRAPS complicated by amyloidosis-related renal failure, treated with the recombinant human interleukin-1 receptor antagonist anakinra at a higher than conventional dosage. This treatment did not present any adverse event and led remarkably to the disappearance of all TRAPS-related manifestations and prompt decrease of laboratory abnormalities, including proteinuria. A review of the medical literature has been also considered to evaluate efficacy and safety of interleukin-1 inhibition in patients with TRAPS.Entities:
Keywords: Anakinra; Autoinflammatory diseases; TNFRSF1A gene; TRAPS; Treatment
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Year: 2017 PMID: 28536823 DOI: 10.1007/s10067-017-3688-4
Source DB: PubMed Journal: Clin Rheumatol ISSN: 0770-3198 Impact factor: 2.980